Aso Kuniyuki, Kono Michihito, Kono Michihiro, Watanabe Toshiyuki, Shimizu Yuka, Ogata Yusuke, Fujieda Yuichiro, Kato Masaru, Oku Kenji, Amengual Olga, Yasuda Shinsuke, Atsumi Tatsuya
Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Lupus. 2020 Sep;29(10):1238-1247. doi: 10.1177/0961203320938453. Epub 2020 Jul 7.
This study aimed to explore the risk factors for 'severe' neuropsychiatric (NP) flare in patients with systemic lupus erythematosus (SLE).
This retrospective study comprised newly diagnosed 184 adult SLE patients who visited Hokkaido University Hospital between 2006 and 2017. In this study, severe NP flare was defined as the occurrence of at least one newly developed British Isles Lupus Assessment Group A score in the neurological domain. Overall severe NP flare-free survival was estimated by Kaplan-Meier analysis. Clinical and demographic profiles at SLE diagnosis were assessed as potential risk items in the adjusted multivariate Cox regression model.
The median follow-up period was 7.9 years (interquartile range (IQR) 4.6-12.3) years. A total of 28 (15.2%) patients had one or more severe NP flares during the observation period. The median time from patient enrolment date to severe NP flare occurrence was 3.1 years (IQR 0.9-6.3 year). The 2- and 10-year severe NP flare-free survival rates were 92.7% and 86.0%, respectively. Among the manifestations of severe NP flare, psychosis was the most frequent (19.1%). In the multivariate model, low serum levels of C4 (hazard ratio (HR) = 3.67, = 0.013) and severe NP manifestations at SLE diagnosis (HR = 7.11, < 0.001) emerged as independent risk factors for developing severe NP flare.
The first severe NP flare presented early in the course of SLE. Low C4 level and severe NP manifestations at SLE diagnosis could predict the development of severe NP flare.
本研究旨在探讨系统性红斑狼疮（SLE）患者发生“严重”神经精神（NP）发作的危险因素。
这项回顾性研究纳入了2006年至2017年间就诊于北海道大学医院的184例新诊断成年SLE患者。在本研究中，严重NP发作被定义为在神经领域至少出现一项新的不列颠群岛狼疮评估组A评分。通过Kaplan-Meier分析估计总体无严重NP发作生存率。在调整后的多变量Cox回归模型中，评估SLE诊断时的临床和人口统计学特征作为潜在风险因素。
中位随访期为7.9年（四分位间距（IQR）4.6 - 12.3年）。在观察期内，共有28例（15.2%）患者发生了一次或多次严重NP发作。从患者入组日期到发生严重NP发作的中位时间为3.1年（IQR 0.9 - 6.3年）。2年和10年无严重NP发作生存率分别为92.7%和86.0%。在严重NP发作的表现中，精神病最为常见（19.1%）。在多变量模型中，血清C4水平低（风险比（HR）= 3.67，= 0.013）和SLE诊断时的严重NP表现（HR = 7.11，< 0.001）是发生严重NP发作的独立危险因素。
首次严重NP发作在SLE病程早期出现。SLE诊断时C4水平低和严重NP表现可预测严重NP发作的发生。
